open access
Does brain degeneration in Wilson disease involve not only copper but also iron accumulation?
Affiliations- II Klinika Neurologii, Instytut Psychiatrii i Neurologii w Warszawie
- Katedra i Zakład Farmakologii Doświadczalnej i Klinicznej, Warszawski Uniwersytet Medyczny
open access
Abstract
Wilson disease (WD) is an autosomal recessive inherited disorder of hepatic copper metabolism. Clinical manifestations of WD include neurologic, hepatic and psychiatric symptoms. Most WD patients with the neuropsychiatric form, and some with the hepatic and presymptomatic forms have both hypointense and hyperintense lesions in basal ganglia on T2-weighted magnetic resonance imaging (MRI), which can be iron and copper accumulation. It has been established that T2* and susceptibility-weighted imaging (SWI) are highly sensitive in demonstrating brain iron accumulation, showing decreased signal intensity. Hypointense globus pallidus (GP) signal has been described on T2-, T2*-weighted images and on SWI as typical MRI lesion for patients with neurodegeneration with brain iron accumulation (NBIA). We investigated whether WD patients have MRI changes suggesting iron accumulation using T2*-weighted and VEN_BOLD SWI imaging protocols.
Material and methodsStandard MRI with additional sequences (T2*-weighted and VEN_BOLD SWI) was performed in consecutively admitted, clinically stable, and treated patients.
ResultsTwenty-eight patients entered the study. Hypointensity in the GP was observed on T2*-weighted images in 10 patients. Using the VEN_BOLD SWI technique, we found hypointense signal in GP in 20 patients.
ConclusionsMRI data suggest not only copper but also iron accumulation in GP in WD patients.
Abstract
Wilson disease (WD) is an autosomal recessive inherited disorder of hepatic copper metabolism. Clinical manifestations of WD include neurologic, hepatic and psychiatric symptoms. Most WD patients with the neuropsychiatric form, and some with the hepatic and presymptomatic forms have both hypointense and hyperintense lesions in basal ganglia on T2-weighted magnetic resonance imaging (MRI), which can be iron and copper accumulation. It has been established that T2* and susceptibility-weighted imaging (SWI) are highly sensitive in demonstrating brain iron accumulation, showing decreased signal intensity. Hypointense globus pallidus (GP) signal has been described on T2-, T2*-weighted images and on SWI as typical MRI lesion for patients with neurodegeneration with brain iron accumulation (NBIA). We investigated whether WD patients have MRI changes suggesting iron accumulation using T2*-weighted and VEN_BOLD SWI imaging protocols.
Material and methodsStandard MRI with additional sequences (T2*-weighted and VEN_BOLD SWI) was performed in consecutively admitted, clinically stable, and treated patients.
ResultsTwenty-eight patients entered the study. Hypointensity in the GP was observed on T2*-weighted images in 10 patients. Using the VEN_BOLD SWI technique, we found hypointense signal in GP in 20 patients.
ConclusionsMRI data suggest not only copper but also iron accumulation in GP in WD patients.
Keywords
Wilson disease, magnetic resonance imaging, copper, iron
About this articleTitle
Does brain degeneration in Wilson disease involve not only copper but also iron accumulation?
Journal
Neurologia i Neurochirurgia Polska
Issue
Pages
542-546
Page views
317
Article views/downloads
704
DOI
10.5114/ninp.2013.39071
Bibliographic record
Neurol Neurochir Pol 2013;47(6):542-546.
Keywords
Wilson disease
magnetic resonance imaging
copper
iron
Authors
Marta Skowrońska
Tomasz Litwin
Karolina Dzieżyc
Agata Wierzchowska
Anna Członkowska
